Deficiencies of carnitine, carnitine acyltransferases, or carnitine/acylcarnitin

Question

Deficiencies of carnitine, carnitine acyltransferases, or carnitine/acylcarnitine translocase

efficiencies of carnitine, carnitine acyltransferases, or carnitine/acylcarnitine translocase affect the metabolism of long-chain fatty acids. Many of the symptoms are similar, and include hypoketotic hypoglycemia (low ketone levels and low blood sugar with fasting), and damage to the liver, heart, or muscles due to fatty acid buildup in those tissues. Symptom severity varies with the form of the deficiency. The following abbreviations are used: carnitine acyltransferase (carnitine palmitoyltransferase), CPT and carnitine/acyl carnitine translocase, CACT. Identify each symptom or effect as a deficiency of CPT I, OPT Il, CACT, or both OPT I and CAOT. One bin will remain empty. CPT I deficiency CPT || deficiency CAOT deficiency CPT I or CAOT deficiency long-chain fatty acids not transferred to carnitine long-chain fatty acids remain attached to carnitine in matrix acyl carnitine not transported across inner mitochondrial membrane

Explanation / Answer

CPT I deficiency= long-chain fatty acids not transferred to carnatine

CPT II deficiency= acyl carnitine not converted to carnitine in matrix

CACT deficiency= acyl carntine not transported into mitocondrial matrix

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